A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature

Abstract

Background: Biliary adenofibroma is an exceptionally rare benign liver tumor with the potential for malignant transformation. In literature, only 21 cases have been described. Clinical presentation: In a healthy 63-year-old woman, a partly solid, partly cystic mass in the left lobe of the liver during a routine ultrasound examination was found. The computed tomography (CT) scan of the abdomen showed a 6.3 × 5.0-cm multilobulated cystic, partly hypervascularized mass in the liver segment IVa, with extension into segments II and IVb. There was no evidence of lymph node or distant metastases. Extirpation of the tumor was indicated by the multidisciplinary tumorboard. Microscopic examination showed a biphasic composed tumor with tubules embedded in fibrous stroma. In addition, there were also areas with pseudopapillary projections, as well as parts with focal cribriform-like growth pattern, which have been indicated as a possible sign of malignant transformation. Additionally, we found two different polymorphisms in the encoded TP53 und KIT in both distinct morphology tumor areas by molecular analysis, which ensured a tumor in malignant transformation. The patient has been alive for 24 months after R0 resection without tumor recurrence. Further investigation of more cases of this rare entity is necessary to proof molecular genesis. Conclusions: We report a rare case of a biliary adenofibroma with transition to an intrahepatic cholangiocellular carcinoma and present a brief literature review.

Imaging features of intrahepatic bile duct adenoma in MRI

“…However, BDA has no history of hepatitis and cirrhosis, and there are no obvious abnormalities in laboratory tests. Most of the enhancements are continuously enhanced without capsules ( 5 , 15 , 16 ). (II) Intrahepatic cholangiocarcinoma: uneven signal, often accompanied by shrinkage of the adjacent liver capsule, atrophy of the hepatic lobes at the distal end of the lesion, and dilatation of the intrahepatic bile duct.…”

“…Because patients usually have no clinical symptoms and are often accompanied by other lesions inside and outside the liver, the lesions are small, and various imaging examinations are not easy to display or obscure, so it is very easy to miss or misdiagnose. BDA may be difficult to distinguish from metastatic carcinomas, particularly welldifferentiated pancreatic ductal adenocarcinoma (3)(4)(5)(6). At present, there are few domestic and foreign literatures, especially in the field of imaging, and most of them are case reports.…”

Imaging presentation of biliary adenofibroma: A case report

“…Currently, although BFs are classified as benign bile duct tumors and precursors by the 2019 World Health Organization tumor classification system[ 5 ], some cases of BF are characterized by malignant transformation, invasion, and even distant metastasis (Tsui et al [ 1 ] first proposed in 1993, postoperative pathology showed malignant transformation in 3 cases[ 6 - 8 ], 3 cases of biliary adenofibroma with invasive carcinoma[ 9 - 11 ], 3 cases were complicated with liver tissue invasion[ 12 - 14 ], 2 cases were complicated with lymph node metastasis[ 15 ], local recurrence occurred in 2 cases[ 16 ]). The clinical manifestations and imaging characteristics of BF are nonspecific.…”

“…Including the present case, 25 cases of BF have been reported to date (Tsui et al [ 1 ] in 1993, Parada et al [ 17 ] in 1997, Haberal et al [ 12 ] in 2001, Akin et al [ 18 ] and Garduño-López et al [ 19 ] in 2002, Gurrera et al [ 20 ] in 2010, Kai et al [ 6 ] and Nguyen et al [ 7 ] in 2012, Jacobs et al [ 13 ] in 2015, Godambe et al [ 9 ]; Thai et al [ 10 ] and Thompson et al [ 15 ] in 2016, Kaminsky et al [ 8 ] and Arnason et al [ 16 ] in 2017, Esteban et al [ 11 ] in 2018, Sturm et al [ 14 ] in 2019), and no differences in sex or age at onset are evident. The mean age at onset is 57.2 ± 17.79 years.…”

Biliary Adenofibroma: A Rare Liver Tumor with Transition to Invasive Carcinoma

“…It has similar histological components as von Meyenburg complexes and is classified as a benign tumor. However, biliary adenofibromas are larger and have the potential for malignant transformation [1][2][3][4][5][6][7]. We report a case of biliary adenofibroma with malignant transformation.…”